1. Haematology revision
Shin Ying Lee

2. 2010
You are on cover for a surgical ward when you are called to Mrs McCulloch, who has just commenced a blood transfusion for a low post operative haemoglobin. Three minutes after the start of the transfusion the patient develops lumbar pains, rigors, dyspnoea and hypotension.

3. 2010
You are on cover for a surgical ward when you are called to Mrs McCulloch, who has just commenced a blood transfusion for a low post operative haemoglobin. Three minutes after the start of the transfusion the patient develops lumbar pains, rigors, dyspnoea and hypotension.

4. What is the name of this complication of the transfusion. 1
Acute hemolytic transfusion reaction

5. What is the most likely procedural reason for this complication to have arisen? 1
ABO incompatibility. Failure to check identity of patient when taking sample for compatibility testing, failure to perform paper identity checks before blood is transfused.

6. List 3 treatments that the patient might then urgently require. 3
High flow oxygen
IV fluids
Diuretics

7. Name 2 further steps that would need to be taken rapidly. 1
1. Send donor blood back to blood bank with notification of event
2. Inform hospital transfusion department immediately

8. What might you detect in the urine? 1
Hemoglobin

9. List three further short term complications of blood transfusions. 3
1. Febrile non-hemolytic reaction
2. Allergic and anaphylactic reaction
3. TRALI (Transfusion related acute lung injury)

10. 2007
A 40 year old woman presents with recent tiredness and lethargy. She has pale conjunctivae and you think there may be some yellowing of the sclera.

11. 2007
A 40 year old woman presents with recent tiredness and lethargy. She has pale conjunctivae and you think there may be some yellowing of the sclera.

12. Name two investigations you would perform and what are you likely to see? 2
1. Bloods – FBC, LFT. high reticulocytes count, High bilirubin levels>50
2. Peripheral blood film- spherocytes, elliptocytes
2. Urine sample – Urobilinogen in the urine

13. What two abnormalities might you expect to see on biochemistry and haematology in haemolytic anaemia? 2
Biochem- High bilirubin levels, high LDH
Haemato- Low hemoglobin, increase reticulocytes count

14. She has a splenectomy. What two things would you want to vaccinate against? 2
Pneumococcal vaccine
Haemophilus influenza type B vaccine

15. What two pieces of advice would you want to give her in regards to her asplenism?2
1) Lifelong prophylactic antibiotics
2) Reimmunisation every 5 years, annual influenza vaccine
3) Increased risk of malaria falciparum, anti-malarial precautions

16. Name two causes of haemolytic anaemia. 2
1) Abnormal membrane (Hereditary spherocytosis, elliptocytosis)
2) Abnormal enzymes (G6PD deficiency)
3) Abnormal hemoglobin synthesis (thalassemia, hemoglobinopathies, sickle cell disease)

17. Leukaemia 4 types Acute myeloid leukaemia (AML)
Acute lymphoid leukaemia (ALL)
Chronic myeloid leukaemia (CML)
Chronic lymphocytic leukaemia (CLL)

18. Investigations
Blood count, White count differentials, red cells, haemoglobin, plaletets
Peripheral blood film
Bone marrow biopsy and aspiration
Immunophenotyping and molecular methods
Cytogenetic analysis

19. CML Anaemia, bruising, unwell
Dragging sensation at the abdomen/ abdomen fullness (splenomegaly)
Translocation of 9: 22. Philadelphia chromosome >80% cases
Chimeric gene (BCR/ABL) > phosphoprotein (p190 and p210) with high tyrosine kinase activity seen.
spectrum of myeloid lineage cells
Breakpoint cluster region (located on chromosome 22q11)Abelson murine leukaemia virus (chromosome 9q34)

20. CML(peripheral blood film)

21. Three phrases
- Chronic phase
- Accelerated phase
- Blast crisis

22. Treatment Imatinib (tyrosine kinase inhibitor) Glivec/Gleevec
Allogenic transplantation

23. CLL
Swollen glands
Constitutional signs and symptoms: Fever, night sweats, weight loss
Mature lymphocytes
Transformed to Diffuse Large B cell lymphoma
Richter’s transformation
Chemotherapy
Stem cell transplant

24. CLL (peripheral blood film)

25. AML
Neoplastic proliferation of blast cells derived from myeloid elements.
Bone marrow aspiration: auer rods, myeloid precursors

27. AML Clinical: >25,000/mm3 FAB type M3,M4eo Mo Auer rods present
Good prognostic factors
Bad prognostic factors
Age
< 50 years
> 60 years
WBC count
<25,000/mm3
>25,000/mm3
FAB type
M3,M4eo Mo
Auer rods
present
absent
Fibrosis

28. ALL

29. ALL Affects children more commonly
Bleeding gums, recurrent infections, tiredness
Hepato-splenomegaly
Bone marrow aspiration: lymphoblast with PAS positive

30. Treatment Chemotherapy
Remission induction : Vincristine, prednisolone, L-asparaginase, daunorubicin
Consolidation
CNS prophylaxis: Intrathecal methotrexate+/- CNS irradiation
Maintenance
Bone marrow transplant

31. Prognostic factors age 4 to 10 years sex girls boys
Clinical:
Good prognostic factors
Bad prognostic factors
age
4 to 10 years
> 10 years, or <2 years
WBC count
<50,000 mm3
>50,000 mm3
Immunophenotype
B-precursor ALL
Mature B or T cell
sex
girls
boys
Chromosomal number
hyperdiploidy
hypodiploidy

32. Lymphoma Two types: Hodgkin’s and Non Hodgkins
Malignancy of the lymph nodes
Diagnosis confirmation – lymph node biopsy
Bloods plus LDH, uric acid
CXR: bihilar lymphadenopathy
Staging: CT scan chest, abdo-pelvis+BMA
B symptoms: fever, weight loss, night sweats, lethargy

33. Types Nodular sclerosis (good prognosis) Mixed cellularity
Lymphocyte rich
Lymphocyte depleted (poor)

34. Reed Sternberg Cell
Nucleus is enclosed with an abundant amphophilic cytoplasm, and contains large, inclusion-like, owl eyed nucleoli, surrounded by a clear halo.
Hodgkin’s Lymphoma

36. Ann Arbor staging
Stage I - a single lymph node area or single extranodal site
Stage II - 2 or more lymph node areas on the same side of the diaphragm
Stage III - denotes lymph node areas on both sides of the diaphragm
Stage IV - disseminated or multiple involvement of the extranodal organs

38. Non Hodgkin’s Treatment
Depending on subtype
Low grade if symptomless none, localized radiotherapy, diffuse (Chlorambucil)
High grade (DLBCL) CHOP
Cychophosphamide
Hydroxydaunorubicin
Oncovin (vincristine)
Prednisolone
+/- Rituximab

39. Myeloma
Malignant clonal proliferation of B-lymphocyte derived plasma cells
Osteolytic bone lesions – unexplained backache, pathological fractures
Anaemic, neutropenia, thrombocytopenia
Renal impairment

40. Investigations
Bloods: Blood count, Hypercalcemia- increase osteoclastic activity
Serum and urine electrophoresis
Urine sample- Bence Jones protein – K, Lambda light chains
B2 microglobulin prognostic test
Skeletal survey- Lytic ‘punched out’ lesion

41. Diagnostic criteria
Monoclonal protein band in serum or urine electrophoresis
Increased plasma cells found on BMB
Evidence of end organ damage from myeloma
- Hypercalcemia
- Renal insufficiency
- Anaemia
- Bone lesions

42. Treatment
Supportive
Chemotherapy

43. Complications of myeloma
Hypercalcaemia
Spinal cord compression
Hyperviscosity
Acute renal failure

44. 4 causes of massive splenomegaly
CML
Malaria
Myelofibrosis
Thalassemia

45. EMQ questions
A 27-year-old man presents with a two-month history of pruritis, fatigue and weight loss. On questioning he admits that whenever he drinks alcohol, he experiences bone pain. On examination he has a rubbery non-tender submandibular lymph node. He has never had infectious mononucleosis.

46. EMQ questions
A 27-year-old man presents with a two-month history of pruritis, fatigue and weight loss. On questioning he admits that whenever he drinks alcohol, he experiences bone pain. On examination he has a rubbery non-tender submandibular lymph node. He has never had infectious mononucleosis.
Hodgkin’s lymphoma

47. EMQ questions
A 68-year-old woman presents with a history of bruising, bone pain and lymphadenopathy. Unbeknownst to the consultant, this patient has a (t9,22) mutation known as the Philadelphia Chromosome. On examination the consultant finds a massively enlarged spleen.

48. EMQ questions
A 68-year-old woman presents with a history of bruising, bone pain and lymphadenopathy. Unbeknownst to the consultant, this patient has a (t9,22) mutation known as the Philadelphia Chromosome. On examination the consultant finds a massively enlarged spleen.
Chronic Myeloid Leukaemia

49. EMQ questions
A 12-year-old girl of Nigerian descent and with a known blood disorder presents to A&E with a two-day history of dyspnoea, cough and fever. You order several investigations and note that she has a Hb of 6g/dl (reference range 11.5 – 1.35 g/dl) and a chest X-ray showing pulmonary infiltrates.

50. EMQ questions
A 12-year-old girl of Nigerian descent and with a known blood disorder presents to A&E with a two-day history of dyspnoea, cough and fever. You order several investigations and note that she has a Hb of 6g/dl (reference range 11.5 – 1.35 g/dl) and a chest X-ray showing pulmonary infiltrates.
Sickle cell anaemia

51. EMQ questions
A 65-year-old lady presents to her GP with a 3-month history of vertigo, tinnitus and visual disturbance. She admits to feeling “a bit down” and the GP decides to carry out some routine bloods. A week later she returns and you note that her blood results show a raised high haemoglobin and a raised pack cell volume and red blood cell count.

52. EMQ questions
A 65-year-old lady presents to her GP with a 3-month history of vertigo, tinnitus and visual disturbance. She admits to feeling “a bit down” and the GP decides to carry out some routine bloods. A week later she returns and you note that her blood results show a raised high haemoglobin and a raised pack cell volume and red blood cell count.
Polycythaemia vera

53. EMQ questions
A 37 year old lady with known hypothyroidism presents to you with fatigue, dyspnoea and palpitations. You note that she is pale and tachycardic. Routine bloods show a macrocytic anaemia. You suspect that this is caused by her hypothyroidism. You find a positive Schilling’s test.

54. EMQ questions
A 37 year old lady with known hypothyroidism presents to you with fatigue, dyspnoea and palpitations. You note that she is pale and tachycardic. Routine bloods show a macrocytic anaemia. You suspect that this is caused by her hypothyroidism. You find a positive Schilling’s test.
Pernicious anaemia

55. Thank you